갑상샘눈병증에서 발병한 눈중증근무력증 환자의 임상양상
The Clinical Features of Ocular Myasthenia Gravis in Thyroid-Associated Ophthalmopathy
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목적: 자가면역 질환인 갑상샘눈병증과 눈중증근무력증은 나타나는 임상양상이 비슷하여 동반될 경우 진단을 간과하기 쉬워 정확한진단을 하기 위해 필요한 임상양상의 특징과 그에 따른 치료경과를 알아보고자 하였다. 대상과 방법: 2002년 1월부터 2012년 12월까지 본원 안과 또는 신경과를 내원하여 갑상샘눈병증과 눈중증근무력증을 함께 진단 받은9명의 의무기록을 후향적으로 조사하여 진단 당시의 증상과 검사 소견을 조사하였다. 결과: 남자 5명, 여자 4명, 평균 연령은 36 ± 16.03세였다. 평균 경과관찰 기간은 45.6개월이었고 9명 중 8명이 그레이브스 병, 1명은하시모토 갑상샘염으로 진단 받았다. 갑상샘눈병증이나 눈중증근무력증의 전형적인 증상이 나타나지 않는 경우가 많았으며, ICE test와 Anti-AchR Ab 역가 검사에서 77.8%로 양성률이 높았다. 눈증증 근무력증 진단 시 시행한 갑상샘 기능 검사에서는 모두 기능 저하또는 항진 소견을 보였다. 추적 소실된 1명을 제외하고 항아세틸콜린에스테라제 제재 또는 스테로이드 치료를 받았던 8명 중 3명은갑상샘눈병증만 먼저 진단이 되어 고용량 스테로이드를 사용하였고, 증상의 호전이 일부만 있어 항아세틸콜린에스테라제 제재를 추가치료 후 호전이 있었다. 결론: 피로에 따른 증상의 변동이 있는 경우, 눈꺼풀 처짐이 동반되는 경우, 치료에도 증상의 호전이 없거나 악화되는 갑상샘눈병증의경우는 눈중증근무력증의 동반여부를 의심하여 관련 검사를 시행 후 적절한 치료를 해야 한다.
Abstract
Purpose: There are some challenges to accurate diagnosis of ocular myasthenia gravis (MG) in thyroid-associated ophthalmopathy(TAO) patients because the clinical features of these diseases are similar. The aim of this study was to discussthe clinical features and treatment options that may help differentiate these 2 diseases. Methods: We performed a retrospective analysis using the medical records of patients who visited our clinic and were diagnosedwith ocular MG and TAO, from January 2002 to December 2012. The diagnosis of Ocular MG was made on the basisof clinical symptoms and signs with laboratory evaluation, including assays for antithyroid and antiacetylcholine receptor(AchRAb) antibodies, and the Ice, neostigmine, and electromyography tests. Results: Of the 9 ocular MG patients with associated ophthalmopathy, 5 were male and 4 were female. The mean age was36 ± 16.0 years and the follow-up period was 45.6 ± 36.6 months. Graves' disease (8 patients) was predominant and all patientsshowed abnormal thyroid function. Atypical symptoms and/or mild clinical features were predominant in ocular MGpatients with TAO. Positive test results were obtained as follows: Neostigmine test 33.3%, electromyography 44.4%, icetest 77.8% and anti-AchR titer test 77.8%. Thyroid function test results were abnormal in all patients. In 3 patients whowere first diagnosed with TAO, symptoms remained persistent despite steroid therapy then improved dramatically by administrationof an anti-acetylcholinesterase agent. These patients were diagnosed with ocular MG in conjunction with TAO. Conclusions: Patients with thyroid disease who show atypical features, symptomatic changes with fatigue, odd appearingptosis, and who, do not exhibit good response to treatment of TAO need to be examined for ocular MG with additional testsand treatment.
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